Lessons for the clinical nephrologist: an uncommon cause of pulmonary-renal syndrome
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LESSONS FOR THE CLINICAL NEPHROLOGIST
Lessons for the clinical nephrologist: an uncommon cause of pulmonary‑renal syndrome Quentin Binet1 · Selda Aydin2 · Jean‑Philippe Lengele1 · Jean‑François Cambier1 Received: 9 July 2020 / Accepted: 17 August 2020 © Italian Society of Nephrology 2020
Abstract Pulmonary-renal syndrome refers to the combination of elevated plasma creatinine concentration and/or abnormal urinalysis with diffuse alveolar hemorrhage, and involves both an urgent diagnostic approach and care. We report the case of a 24-yearold man presenting with diffuse alveolar hemorrhage as well as a nephritic syndrome associating kidney failure, moderate hypertension, hematuria and selective glomerular proteinuria. The initial high suspicion of anti-glomerular basement membrane (GBM) disease or ANCA-associated vasculitis justified intravenous pulse-corticotherapy in association with plasma exchange. Renal biopsy was remarkable for an IgA nephropathy, lesions of active thrombotic microangiopathy (TMA) and a positive staining for complement factor C4d. Because anti-GBM and ANCA antibodies returned negative, plasma exchange was discontinued, but oral corticosteroids were maintained to prevent alveolar hemorrhage recurrence. In the absence of renal function recovery, hemodialysis was initiated. TMA lesions are frequently seen in IgA nephropathy and are associated with a poorer prognosis. Complement activation seems to be involved in the development of those lesions and contributes to disease progression. Conversely, alveolar hemorrhage in the setting of IgA nephropathy is uncommon. It is thought to result from non-specific mucosal hemorrhage, an immune complex mediated basement membrane damage and an IgA-mediated capillaritis against basement membrane antigens. Keywords Pulmonary-renal syndrome · IgA nephropathy · Alveolar hemorrhage · Thrombotic microangiopathy A 24-year-old male presented to the emergency department with acute respiratory distress, hemoptysis and moderate hypertension. His past medical history included fluctuating macroscopic hematuria, and his last measured creatinine level was 1.04 mg/dL 3.5 years ago. He smokes 15 cigarettes a day. Family history and physical examination were noncontributory. Laboratory tests showed severe inflammatory syndrome (CRP 171 mg/L), non-hemolytic anemia (9.0 g/ dL) and kidney injury (creatinine 9.5 mg/dL and normal kidney size on abdominal computed tomography) associated with hematuria (500 RBC/µL with casts) and selective glomerular proteinuria (24-h proteinuria of 1.9 g, mostly albumin). Coagulation tests were normal. Serological studies returned within reference range or negative, including * Quentin Binet [email protected] 1
Department of Nephrology, Grand Hôpital de Charleroi, Gilly, Belgium
Department of Pathology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
2
antinuclear antibodies (ANA), anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane (GBM) antibodies, complement levels, and viral serolo
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